POS0945 ILD PROGRESSION ON STANDARD OF CARE VARIES IN PATIENTS WITH CONNECTIVE TISSUE DISEASE
Authors: E. Langballe, P. P. Diep, H. Andersson, C. Bruni, H. Fretheim, T. Garen, R. Gunnarsson, Ø. Midtvedt, Ø. Molberg, Ø. Palm, S. Reiseter, T. M. Aaløkken, O. Distler, A. M. Hoffmann-Vold
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> A subset of patients with connective tissue disease associated interstitial lung disease (CTD-ILD) progresses. These patients have been included in clinical ILD trials as a group despite their different underlying diseases. It has not been studied whether this basket approach of pooling different CTD-ILDs is valid, e.g., if the disease course of the ILDs depends on the underlying CTD. In addition, the impact of immunosuppressive treatment, which CTD-ILD patients receive for ILD or other organ manifestations as a standard of care, is not fully understood. <h3>Objectives</h3> To assess ILD progression in patients with CTD-ILDs on standard of care treatment. <h3>Methods</h3> CTD-ILD patients (N=504) from a Norwegian and Swiss cohort fulfilling the respective classification criteria of the underlying CTD with ILD diagnosed on HRCT were included. Data were extracted from prospectively collected local registries. Additional data were extracted from electronic patient records. Change in absolute FVC% predicted over 12 months was calculated. In addition, ILD progression was assessed over 12 months on standard of care treatment, defined as: (I) Absolute decline of FVC (% predicted) ≥10% (II) 2022 ATS/ERS/JRS/ALAT guideline criteria including 2/3 of (1) worsening of respiratory symptoms; (2) absolute decline in FVC ≥ 5% or in DLCO ≥ 10% and (3) disease progression on HRCT over 12 months Multivariable logistic regression and descriptive statistics were applied. Analyses were adjusted for underlying CTD and immunosuppressive treatment (including corticosteroids any dose, csDMARDs, mycophenolate, cyclophosphamide, CD-20/TNF/ JAK/IL6-inhibitors). <h3>Results</h3> The total CTD-ILD cohort of 504 patients included 231 (46%) systemic sclerosis (SSc)-ILD, 91 (19%) anti-synthetase syndrome (ASS)-ILD, 82 (16%) rheumatoid arthritis (RA)-ILD, 57 (11%) primary Sjögren syndrome (pSS)-ILD and 43 (9%) mixed connective tissue disease (MCTD)-ILD patients (Table 1). Mean absolute change in FVC% over 12 months was for the entire cohort -0,74% (range -34 to +42%), 57 (13,7%) had an FVC decline ≥10% and 47 (12%) fulfilled the guideline criteria. ILD progression varied between the diagnoses, with SSc-ILD and pSS-ILD showing the strongest absolute FVC decline over 12 months and the highest percentage of progressive patients defined by the 2022 guideline. Additionally, patients with SSc-ILD and MCTD-ILD had more frequent FVC ≥10% events (Table 1). Overall, 240 (62,8%) patients were treated with immunosuppressives (table 1). There was no difference between patients treated and not treated in absolute FVC decline (OR 1.00, 95%CI 0.98-1.02, p= 0.705), and in the percentage of progressive patients according to the guideline criteria (OR 1.00, 95%CI 0.54-1.86, p= 0.995). However, there was less FVC decline ≥10% (OR 0.51, 95% CI 0.28-0.90, p= 0.022) in patients treated with immunosuppressives. Patients with SSc-ILD (11% vs 20%, p=0.059) and pSS-ILD (8% vs 19%, p=0.329) showed numerically less FVC decline ≥10%. The different CTD diagnoses had different patterns of treatment with immunosuppressives, with the majority of RA and ASS-ILD being treated (Table 1). <h3>Conclusion</h3> In this CTD-ILD cohort, both underlying CTD and immunosuppressive treatment seemed to be associated with different patterns of ILD progression over a 12-month period. This has implications for the enrichment for progressive disease and inclusion of CTD-ILD patients into clinical trials. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> Emily Langballe: None declared, Phuong Phuong Diep Speakers bureau: Boehringer-Ingelheim, Consultant of: Boehringer-Ingelheim, Helena Andersson: None declared, Cosimo Bruni Speakers bureau: Eli-Lilly, Consultant of: Boehringer Ingelheim, Grant/research support from: Gruppo Italiano Lotta alla Sclerodermia (GILS), European Scleroderma Trials and Research Group (EUSTAR), Foundation for research in Rheumatology (FOREUM), Scleroderma Clinical Trials Consortium (SCTC). Educational grants from AbbVie, Håvard Fretheim Speakers bureau: Boehringer Ingelheim, Grant/research support from: Jannsen, Torhild Garen: None declared, Ragnar Gunnarsson: None declared, Øyvind Midtvedt: None declared, Øyvind Molberg: None declared, Øyvind Palm: None declared, Silje Reiseter: None declared, Trond M Aaløkken: None declared, Oliver Distler Speakers bureau: 4PPharma, Abbvie, Acceleron, Alcimed, Altavant, Amgen, AnaMar, Arxx, AstraZeneca, Baecon, Blade, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, Galderma, Galapagos, Glenmark, Gossamer, iQvia, Horizon, Inventiva, Janssen, Kymera, Lupin, Medscape, Merck, Miltenyi Biotec, Mitsubishi Tanabe, Novartis, Prometheus, Redxpharma, Roivant, Sanofi and Topadur., Consultant of: 4PPharma, Abbvie, Acceleron, Alcimed, Altavant, Amgen, AnaMar, Arxx, AstraZeneca, Baecon, Blade, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, Galderma, Galapagos, Glenmark, Gossamer, iQvia, Horizon, Inventiva, Janssen, Kymera, Lupin, Medscape, Merck, Miltenyi Biotec, Mitsubishi Tanabe, Novartis, Pfizer, Prometheus, Redxpharma, Roivant, Sanofi and Topadur, Grant/research support from: Kymera, Mitsubishi Tanabe, Boehringer Ingelheim, Anna-Maria Hoffmann-Vold Speakers bureau: Boehringer Ingelheim, Jannsen, Medscape, Merck Sharp &amp; Dohme and Roche, Consultant of: ARXX, Boehringer Ingelheim, Genentech, Jannsen, Medscape, Merck Sharp &amp; Dohme and Roche, Grant/research support from: Boehringer Ingelheim, Jannsen.